staphylococcal scalded skin syndrome
Sunday 5 February 2006
Staphylococcal scalded skin syndrome (SSSS), also called Ritter disease, is caused by the exfoliative A and B toxins of Staphylococcus aureus.
SSSS is an exfoliative dermatitis that most frequently occurs in children with staphylococcal infections of the nasopharynx or skin. In staphylococcal scalded skin syndrome, there is a sunburnlike rash that spreads over the entire body and forms fragile bullae that lead to partial or total skin loss.
The intraepithelial split in staphylococcal scalded skin syndrome is in the granulosa layer, distinguishing it from toxic epidermal necrolysis, or Lyell’s disease, which is secondary to drug hypersensitivity and causes splitting at the epidermal-dermal junction.
The exfoliative toxins produced by S. aureus are serine proteases that split the skin by cleaving the protein desmoglein-1 (DSG1), a member of the desmoglein family (DSGs), that are an important component of epidermal desmosomes that hold epidermal cells tightly together (see desmosomal proteins).
This can cause the superficial epidermis to split away from the deeper skin, making the patient vulnerable to secondary infections. Exfoliation can occur at the site of staphylococcal skin infection (bullous impetigo) or can be widespread, when secreted toxin from a localized infection causes disseminated loss of the superficial epidermis (staphylococcal scalded-skin syndrome).
Payne AS, Hanakawa Y, Amagai M, Stanley JR. Desmosomes and disease: pemphigus and bullous impetigo. Curr Opin Cell Biol. 2004 Oct;16(5):536-43. PMID: 15363804